Etiologic and diagnostic facets of Creutzfeldt-Jakob disease: The effect of genes and environment

textabstractTransmissible spongiform encephalopathies (TSE) or prion diseases constitute a fascinating group of neurological disorders. In spite of their rarity, their unique etiopathological mechanisms and epidemiological aspects have attracted a considerable number of researchers. This thesis focuses on two facets: etiological mechanisms and diagnostic tests of Creutzfeldt-Jakob disease (CJD). We have searched new insights into these two topics using genetic and classic epidemiological methodology. For the studies presented in this book, we have used data from the Dutch, Italian and UK CJD National Surveillance Systems, the 'European collaborative Study group of CJD' (EUROCJD), the 'extended European collaborative Study group of CJD' (NEUROCJD), the 'Early clinical diagnosis of human spongiform encephalopathies by analysis of biological fluids-CJD Markers collaboration' (EPICJD), and the 'Rotterdam Study'